This booklet is for people who have systemic lupus erythematosus, commonly
called SLE or lupus, as well as for their family and friends and others who
want to better understand the disease. The booklet describes the disease and
its symptoms and contains information about diagnosis and treatment as well
as current research efforts supported by the National Institute of Arthritis
and Musculoskeletal and Skin Diseases (NIAMS) and other components of the Department
of Health and Human Services' National Institutes of Health (NIH). It also
discusses issues such as health care, pregnancy, and quality of life for people
with lupus. If you have further questions after reading this booklet, you may
wish to discuss them with your doctor.

Lupus is one of many disorders of the immune system known as autoimmune diseases.
In autoimmune diseases, the immune system turns against parts of the body it
is designed to protect. This leads to inflammation and damage to various body
tissues. Lupus can affect many parts of the body, including the joints, skin,
kidneys, heart, lungs, blood vessels, and brain. Although people with the disease
may have many different symptoms, some of the most common ones include extreme
fatigue, painful or swollen joints (arthritis), unexplained fever, skin rashes,
and kidney problems.

At present, there is no cure for lupus. However, lupus can be effectively
treated with drugs, and most people with the disease can lead active, healthy
lives. Lupus is characterized by periods of illness, called flares, and periods
of wellness, or remission. Understanding how to prevent flares and how to treat
them when they do occur helps people with lupus maintain better health. Intense
research is underway, and scientists funded by the NIH are continuing to make
great strides in understanding the disease, which may ultimately lead to a
cure.

Two of the major questions researchers are studying are who gets lupus and
why. We know that many more women than men have lupus. Lupus is three times
more common in African American women than in Caucasian women and is also more
common in women of Hispanic, Asian, and Native American descent. In addition,
lupus can run in families, but the risk that a child or a brother or sister
of a patient will also have lupus is still quite low. It is difficult to estimate
how many people in the United States have the disease because its symptoms
vary widely and its onset is often hard to pinpoint.

There are several kinds of lupus:

Systemic lupus erythematosus (SLE) is the form of the disease that most
people are referring to when they say "lupus." The word "systemic" means
the disease can affect many parts of the body. The symptoms of SLE may be
mild or serious. Although SLE usually first affects people between the ages
of 15 and 45 years, it can occur in childhood or later in life as well. This
booklet focuses on SLE.

Discoid lupus erythematosus is a chronic skin disorder in which a red,
raised rash appears on the face, scalp, or elsewhere. The raised areas may
become thick and scaly and may cause scarring. The rash may last for days
or years and may recur. A small percentage of people with discoid lupus have
or develop SLE later.

Subacute cutaneous lupus erythematosus refers to skin lesions that appear
on parts of the body exposed to sun. The lesions do not cause scarring.

Drug-induced lupus is a form of lupus caused by medications. Many different
drugs can cause drug-induced lupus. Symptoms are similar to those of SLE
(arthritis, rash, fever, and chest pain) and they typically go away completely
when the drug is stopped. The kidneys and brain are rarely involved.

Neonatal lupus is a rare disease that can occur in newborn babies of women
with SLE, Sjögren's syndrome, or no disease at all. Scientists suspect that
neonatal lupus is caused by autoantibodies in the mother's blood called anti-Ro
(SSA) and anti-La (SSB). Autoantibodies ("auto" means self) are blood proteins
that act against the body's own parts. At birth, the babies have a skin rash,
liver problems, and low blood counts. These symptoms gradually go away over
several months. In rare instances, babies with neonatal lupus may have a
serious heart problem that slows down the natural rhythm of the heart. Neonatal
lupus is rare, and most infants of mothers with SLE are entirely healthy.
All women who are pregnant and known to have anti-Ro (SSA) or anti-La (SSB)
antibodies should be monitored by echocardiograms (a test that monitors the
heart and surrounding blood vessels) during the 16th and 30th weeks of pregnancy.
It is important for women with SLE or other related autoimmune disorders
to be under a doctor's care during pregnancy. Physicians can now identify
mothers at highest risk for complications, allowing for prompt treatment
of the infant at or before birth. SLE can also flare during pregnancy, and
prompt treatment can keep the mother healthier longer.

Understanding What Causes Lupus

Lupus is a complex disease, and its cause is unknown. It is likely that a
combination of genetic, environmental, and possibly hormonal factors work together
to cause the disease. Scientists are making progress in understanding lupus,
as described here and in the "Current Research" section of this booklet. The
fact that lupus can run in families indicates that its development has a genetic
basis. Recent research suggests that genetics plays an important role; however,
no specific "lupus gene" has been identified yet. Studies suggest that several
different genes may be involved in determining a person's likelihood of developing
the disease, which tissues and organs are affected, and the severity of disease.
However, scientists believe that genes alone do not determine who gets lupus
and that other factors also play a role. Some of the factors scientists are
studying include sunlight, stress, certain drugs, and infectious agents such
as viruses.

In lupus, the body's immune system does not work as it should. A healthy immune
system produces proteins called antibodies and specific cells called lymphocytes
that help fight and destroy viruses, bacteria, and other foreign substances
that invade the body. In lupus, the immune system produces antibodies against
the body's healthy cells and tissues. These antibodies, called autoantibodies,
contribute to the inflammation of various parts of the body and can cause damage
to organs and tissues. The most common type of autoantibody that develops in
people with lupus is called an antinuclear antibody (ANA) because it reacts
with parts of the cell's nucleus (command center). Doctors and scientists do
not yet understand all of the factors that cause inflammation and tissue damage
in lupus, and researchers are actively exploring them.

Each person with lupus has slightly different symptoms that can range from
mild to severe and may come and go over time. However, some of the most common
symptoms of lupus include painful or swollen joints (arthritis), unexplained
fever, and extreme fatigue. A characteristic red skin rash-the so-called butterfly
or malar rash-may appear across the nose and cheeks. Rashes may also occur
on the face and ears, upper arms, shoulders, chest, and hands. Because many
people with lupus are sensitive to sunlight (called photosensitivity), skin
rashes often first develop or worsen after sun exposure.

Pale or purple fingers or toes from cold or stress (Raynaud's phenomenon)

Sensitivity to the sun

Swelling (edema) in legs or around eyes

Mouth ulcers

Swollen glands

Extreme fatigue

Other symptoms of lupus include chest pain, hair loss, anemia (a decrease
in red blood cells), mouth ulcers, and pale or purple fingers and toes from
cold and stress. Some people also experience headaches, dizziness, depression,
confusion, or seizures. New symptoms may continue to appear years after the
initial diagnosis, and different symptoms can occur at different times. In
some people with lupus, only one system of the body, such as the skin or joints,
is affected. Other people experience symptoms in many parts of their body.
Just how seriously a body system is affected varies from person to person.
The following systems in the body also can be affected by lupus.

Kidneys: Inflammation of the kidneys (nephritis) can impair their ability
to get rid of waste products and other toxins from the body effectively.
There is usually no pain associated with kidney involvement, although some
patients may notice swelling in their ankles. Most often, the only indication
of kidney disease is an abnormal urine or blood test. Because the kidneys
are so important to overall health, lupus affecting the kidneys generally
requires intensive drug treatment to prevent permanent damage.

Lungs: Some people with lupus develop pleuritis, an inflammation of the
lining of the chest cavity that causes chest pain, particularly with breathing.
Patients with lupus also may get pneumonia.

Blood vessels: Blood vessels may become inflamed (vasculitis), affecting
the way blood circulates through the body. The inflammation may be mild and
may not require treatment or may be severe and require immediate attention.

Blood: People with lupus may develop anemia, leukopenia (a decreased number
of white blood cells), or thrombocytopenia (a decrease in the number of platelets
in the blood, which assist in clotting). Some people with lupus may have
an increased risk for blood clots.

Heart: In some people with lupus, inflammation can occur in the heart itself
(myocarditis and endocarditis) or the membrane that surrounds it (pericarditis),
causing chest pains or other symptoms. Lupus can also increase the risk of
atherosclerosis (hardening of the arteries).

Diagnosing lupus can be difficult. It may take months or even years for doctors
to piece together the symptoms to diagnose this complex disease accurately.
Making a correct diagnosis of lupus requires knowledge and awareness on the
part of the doctor and good communication on the part of the patient. Giving
the doctor a complete, accurate medical history (for example, what health problems
you have had and for how long) is critical to the process of diagnosis. This
information, along with a physical examination and the results of laboratory
tests, helps the doctor consider other diseases that may mimic lupus, or determine
if the patient truly has the disease. Reaching a diagnosis may take time as
new symptoms appear.

No single test can determine whether a person has lupus, but several laboratory
tests may help the doctor to make a diagnosis. The most useful tests identify
certain autoantibodies often present in the blood of people with lupus. For
example, the antinuclear antibody (ANA) test is commonly used to look for autoantibodies
that react against components of the nucleus, or "command center," of the body's
cells. Most people with lupus test positive for ANA; however, there are a number
of other causes of a positive ANA besides lupus, including infections, other
autoimmune diseases, and occasionally as a finding in healthy people. The ANA
test simply provides another clue for the doctor to consider in making a diagnosis.
In addition, there are blood tests for individual types of autoantibodies that
are more specific to people with lupus, although not all people with lupus
test positive for these and not all people with these antibodies have lupus.
These antibodies include anti-DNA, anti-Sm, anti-RNP, anti-Ro (SSA), and anti-La
(SSB). The doctor may use these antibody tests to help make a diagnosis of
lupus.

Some tests are used less frequently but may be helpful if the cause of a person's
symptoms remains unclear. The doctor may order a biopsy of the skin or kidneys
if those body systems are affected. Some doctors may order a test for anticardiolipin
(or antiphospholipid) antibody. The presence of this antibody may indicate
increased risk for blood clotting and increased risk for miscarriage in pregnant
women with lupus. Again, all these tests merely serve as tools to give the
doctor clues and information in making a diagnosis. The doctor will look at
the entire picture-medical history, symptoms, and test results-to determine
if a person has lupus.

Other laboratory tests are used to monitor the progress of the disease once
it has been diagnosed. A complete blood count, urinalysis, blood chemistries,
and the erythrocyte sedimentation rate (ESR) test can provide valuable information.
Another common test measures the blood level of a group of substances called
complement. People with lupus often have increased ESRs and low complement
levels, especially during flares of the disease. X rays and other imaging tests
can help doctors see the organs affected by SLE.

Diagnosing and treating lupus are often a team effort between the patient
and several types of health care professionals. A person with lupus can go
to his or her family doctor or internist, or can visit a rheumatologist. A
rheumatologist is a doctor who specializes in rheumatic diseases (arthritis
and other inflammatory disorders, often involving the immune system). Clinical
immunologists (doctors specializing in immune system disorders) may also treat
people with lupus. As treatment progresses, other professionals often help.
These may include nurses, psychologists, social workers, nephrologists (doctors
who treat kidney disease), hematologists (doctors specializing in blood disorders),
dermatologists (doctors who treat skin disease), and neurologists (doctors
specializing in disorders of the nervous system).

The range and effectiveness of treatments for lupus have increased dramatically,
giving doctors more choices in how to manage the disease. It is important for
the patient to work closely with the doctor and take an active role in managing
the disease. Once lupus has been diagnosed, the doctor will develop a treatment
plan based on the patient's age, sex, health, symptoms, and lifestyle. Treatment
plans are tailored to the individual's needs and may change over time. In developing
a treatment plan, the doctor has several goals: to prevent flares, to treat
them when they do occur, and to minimize organ damage and complications. The
doctor and patient should reevaluate the plan regularly to ensure it is as
effective as possible.

NSAIDs: For people with joint or chest pain or fever, drugs
that decrease inflammation, called nonsteroidal anti-inflammatory drugs (NSAIDs),
are often used. While some NSAIDs, such as ibuprofen and naproxen, are available
over the counter, a doctor's prescription is necessary for others. NSAIDs may
be used alone or in combination with other types of drugs to control pain,
swelling, and fever. Even though some NSAIDs may be purchased without a prescription,
it is important that they be taken under a doctor's direction. Common side
effects of NSAIDs can include stomach upset, heartburn, diarrhea, and fluid
retention. Some people with lupus also develop liver, kidney, or even neurological
complications, making it especially important to stay in close contact with
the doctor while taking these medications.

Antimalarials: Antimalarials are another type of drug commonly
used to treat lupus. These drugs were originally used to treat malaria, but
doctors have found that they also are useful for lupus. A common antimalarial
used to treat lupus is hydroxychloroquine (Plaquenil)*. It may be used alone
or in combination with other drugs and generally is used to treat fatigue,
joint pain, skin rashes, and inflammation of the lungs. Clinical studies have
found that continuous treatment with antimalarials may prevent flares from
recurring. Side effects of anti-malarials can include stomach upset and, extremely
rarely, damage to the retina of the eye.

* Brand names included in this publication are provided as examples
only, and their inclusion does not mean that these products are endorsed
by the National Institutes of Health or any other Government agency. Also,
if a particular brand name is not mentioned, this does not mean or imply
that the product is unsatisfactory.

Corticosteroids: The mainstay of lupus treatment involves
the use of corticosteroid hormones, such as prednisone (Deltasone), hydrocortisone,
methylprednisolone (Medrol), and dexamethasone (Decadron, Hexadrol). Corticosteroids
are related to cortisol, which is a natural anti-inflammatory hormone. They
work by rapidly suppressing inflammation. Corticosteroids can be given by mouth,
in creams applied to the skin, or by injection. Because they are potent drugs,
the doctor will seek the lowest dose with the greatest benefit. Short-term
side effects of corticosteroids include swelling, increased appetite, and weight
gain. These side effects generally stop when the drug is stopped. It is dangerous
to stop taking corticosteroids suddenly, so it is very important that the doctor
and patient work together in changing the corticosteroid dose. Sometimes doctors
give very large amounts of corticosteroid by vein over a brief period of time
(days) ("bolus" or "pulse" therapy). With this treatment, the typical side
effects are less likely and slow withdrawal is unnecessary.

Long-term side effects of corticosteroids can include stretch marks on the
skin, weakened or damaged bones (osteoporosis and osteonecrosis), high blood
pressure, damage to the arteries, high blood sugar (diabetes), infections,
and cataracts. Typically, the higher the dose and the longer they are taken,
the greater the risk and severity of side effects. Researchers are working
to develop ways to limit or offset the use of corticosteroids. For example,
corticosteroids may be used in combination with other, less potent drugs, or
the doctor may try to slowly decrease the dose once the disease is under control.
People with lupus who are using corticosteroids should talk to their doctors
about taking supplemental calcium and vitamin D or other drugs to reduce the
risk of osteoporosis (weakened, fragile bones).

Immunosuppressives: For some patients whose kidneys or central
nervous systems are affected by lupus, a type of drug called an immunosuppressive
may be used. Immunosuppressives, such as cyclophosphamide (Cytoxan) and mycophenolate
mofetil (CellCept), restrain the overactive immune system by blocking the production
of immune cells. These drugs may be given by mouth or by infusion (dripping
the drug into the vein through a small tube). Side effects may include nausea,
vomiting, hair loss, bladder problems, decreased fertility, and increased risk
of cancer and infection. The risk for side effects increases with the length
of treatment. As with other treatments for lupus, there is a risk of relapse
after the immunosuppressives have been stopped.

Other Therapies: In some patients, methotrexate (Folex, Mexate,
Rheumatrex), a disease-modifying antirheumatic drug, may be used to help control
the disease. Working closely with the doctor helps ensure that treatments for
lupus are as successful as possible. Because some treatments may cause harmful
side effects, it is important to report any new symptoms to the doctor promptly.
It is also important not to stop or change treatments without talking to the
doctor first.

Alternative and Complementary Therapies: Because of the nature
and cost of the medications used to treat lupus and the potential for serious
side effects, many patients seek other ways of treating the disease. Some alternative
approaches people have tried include special diets, nutritional supplements,
fish oils, ointments and creams, chiropractic treatment, and homeopathy. Although
these methods may not be harmful in and of themselves, and may be associated
with symptomatic or psychosocial benefit, no research to date shows that they
affect the disease process or prevent organ damage. Some alternative or complementary
approaches may help the patient cope or reduce some of the stress associated
with living with a chronic illness. If the doctor feels the approach has value
and will not be harmful, it can be incorporated into the patient's treatment
plan. However, it is important not to neglect regular health care or treatment
of serious symptoms. An open dialogue between the patient and physician about
the relative values of complementary and alternative therapies allows the patient
to make an informed choice about treatment options.

Despite the symptoms of lupus and the potential side-effects of treatment,
people with lupus can maintain a high quality of life overall. One key to managing
lupus is to understand the disease and its impact. Learning to recognize the
warning signs of a flare can help the patient take steps to ward it off or
reduce its intensity. Many people with lupus experience increased fatigue,
pain, a rash, fever, abdominal discomfort, headache, or dizziness just before
a flare. Developing strategies to prevent flares can also be helpful, such
as learning to recognize your warning signals and maintaining good communication
with your doctor.

It is also important for people with lupus to receive regular health care,
instead of seeking help only when symptoms worsen. Results from a medical exam
and laboratory work on a regular basis allows the doctor to note any changes
and to identify and treat flares early. The treatment plan, which is tailored
to the individual's specific needs and circumstances, can be adjusted accordingly.
If new symptoms are identified early, treatments may be more effective. Other
concerns also can be addressed at regular checkups. The doctor can provide
guidance about such issues as the use of sunscreens, stress reduction, and
the importance of structured exercise and rest, as well as birth control and
family planning. Because people with lupus can be more susceptible to infections,
the doctor may recommend yearly influenza vaccinations or pneumococcal vaccinations
for some patients.

Women with lupus should receive regular preventive health care, such as gynecological
and breast examinations. Men with lupus should have the prostate-specific antigen
(PSA) test. Both men and women need to have their blood pressure and cholesterol
checked on a regular basis. If a person is taking corticosteroids or antimalarial
medications, an eye exam should be done at least yearly to screen for and treat
eye problems.

Staying healthy requires extra effort and care for people with lupus, so it
becomes especially important to develop strategies for maintaining wellness.
Wellness involves close attention to the body, mind, and spirit. One of the
primary goals of wellness for people with lupus is coping with the stress of
having a chronic disorder. Effective stress management varies from person to
person. Some approaches that may help include exercise, relaxation techniques
such as meditation, and setting priorities for spending time and energy.

Developing and maintaining a good support system is also important. A support
system may include family, friends, medical professionals, community organizations,
and support groups. Participating in a support group can provide emotional
help, boost self-esteem and morale, and help develop or improve coping skills.
(For more information on support groups, see the "Additional Resources" section
at the end of this booklet.)

Learning more about lupus may also help. Studies have shown that patients
who are well-informed and participate actively in their own care experience
less pain, make fewer visits to the doctor, build self-confidence, and remain
more active.

Although a lupus pregnancy is considered high risk, most women with lupus
carry their babies safely to the end of their pregnancy. Women with lupus have
a higher rate of miscarriage and premature births compared with the general
population. In addition, women who have antiphospholipid antibodies are at
a greater risk of miscarriage in the second trimester because of their increased
risk of blood clotting in the placenta. Lupus patients with a history of kidney
disease have a higher risk of preeclampsia (hypertension with a buildup of
excess watery fluid in cells or tissues of the body). Pregnancy counseling
and planning before pregnancy are important. Ideally, a woman should have no
signs or symptoms of lupus and be taking no medications for at least 6 months
before she becomes pregnant.

Some women may experience a mild to moderate flare during or after their pregnancy;
others do not. Pregnant women with lupus, especially those taking corticosteroids,
also are more likely to develop high blood pressure, diabetes, hyperglycemia
(high blood sugar), and kidney complications, so regular care and good nutrition
during pregnancy are essential. It is also advisable to have access to a neonatal
(newborn) intensive care unit at the time of delivery in case the baby requires
special medical attention.

Lupus is the focus of intense research as scientists try to determine what
causes the disease and how it can best be treated. Some of the questions they
are working to answer include: Why are women more likely than men to have the
disease? Why are there more cases of lupus in some racial and ethnic groups?
What goes wrong in the immune system, and why? How can we correct the way the
immune system functions once something goes wrong? What treatment approaches
will work best to lessen lupus symptoms? How do we cure lupus?

To help answer these questions, scientists are developing new and better ways
to study the disease. They are doing laboratory studies that compare various
aspects of the immune systems of people with lupus with those of other people
both with and without lupus. They also use mice with disorders resembling lupus
to better understand the abnormalities of the immune system that occur in lupus
and to identify possible new therapies.

The National Institute of Arthritis and Musculoskeletal and Skin Diseases
(NIAMS), a component of the Department of Health and Human Services' National
Institutes of Health (NIH), has a major focus on lupus research in its on campus
program in Bethesda, Maryland. By evaluating patients with lupus and their
relatives, researchers oncampus are learning more about how lupus develops
and changes over time. The NIAMS also funds many lupus researchers across the
United States. Some of these researchers are studying the genetic factors that
increase a person's risk for developing lupus. To help scientists gain new
knowledge, the NIAMS also has established Specialized Centers of Research devoted
specifically to lupus research. In addition, the NIAMS is funding lupus registries
that gather medical information as well as blood and tissue samples from patients
and their relatives. This gives researchers across the country access to information
and materials they can use to help identify genes that determine susceptibility
to the disease.

Identifying genes that play a role in the development of lupus is an active
area of research. For example, researchers suspect that a genetic defect in
a cellular process called apoptosis, or "programmed cell death," exists in
people with lupus. Apoptosis is similar to the process that causes leaves to
turn color in autumn and fall from trees; it allows the body to eliminate cells
that have fulfilled their function and typically need to be replaced. If there
is a problem in the apoptosis process, harmful cells may stay around and do
damage to the body's own tissues. For example, in a mutant mouse strain that
develops a lupus-like illness, one of the genes that controls apoptosis is
defective. When it is replaced by a normal gene, the mice no longer develop
signs of the disease. Scientists are studying what role genes involved in apoptosis
may play in human disease development.

Studying genes for complement, a series of proteins in the blood that play
an important part in the immune system, is another active area of lupus research.
Complement acts as a backup for antibodies, helping them destroy foreign substances
that invade the body. If there is a decrease in complement, the body is less
able to fight or destroy foreign substances. If these substances are not removed
from the body, the immune system may become overactive and begin to make autoantibodies.

Recent large studies of families with lupus have identified a number of genetic
regions that appear to be associated with risk of SLE. Although the specific
genes and their function remain unknown, intensive work in mapping the entire
human genome offers promise that these genes will be identified in the near
future. This should provide knowledge of the complex factors that contribute
to lupus susceptibility.

NIAMS-funded researchers are uncovering the impact of genetic, socioeconomic,
and cultural factors on the course and outcome of lupus in Hispanics, African
Americans, and Caucasians. Preliminary data show that African American and
Hispanic lupus patients typically have more kidney damage compared with Caucasians.
In addition, NIAMS-funded researchers found that African American lupus patients
have more skin damage compared with Hispanics and Caucasians, and that the
death rate from lupus is higher in African Americans and Hispanics compared
with Caucasians.

It is thought that autoimmune diseases, such as lupus, occur when a genetically
susceptible individual encounters an unknown environmental agent or trigger.
In this circumstance, an abnormal immune response can be initiated that leads
to the signs and symptoms of lupus. Research has focused on both the genetic
susceptibility and the environmental trigger. Although the environmental trigger
remains unknown, microbial agents such as Epstein-Barr virus and others have
been considered. Researchers also are studying other factors that may affect
a person's susceptibility to lupus. For example, because lupus is more common
in women than in men, some researchers are investigating the role of hormones
and other male-female differences in the development and course of the disease.
A current study funded by the NIH is focusing on the safety and effectiveness
of oral contraceptives (birth-control pills) and hormone replacement therapy
in women with lupus. Doctors have worried about the wisdom of prescribing oral
contraceptives or estrogen replacement therapy for women with lupus because
of a widely held view that estrogens can make the disease worse. Oral contraceptives
and estrogen replacement therapy do not, as once feared, appear to intensify
lupus symptoms. Scientists do not know the effects of oral contraceptives on
women with antiphospholipid antibody syndrome.

Patients with lupus are at risk of developing atherosclerotic vascular disease
(hardening of the blood vessels that can cause heart attack, angina, or stroke).
The increased risk is due partly to having lupus and partly to steroid therapy.
Preventing atherosclerotic vascular disease in lupus patients is a new area
of study. NIAMS-funded researchers are studying the most effective ways to
manage cardiovascular risk factors and prevent cardiovascular disease in adult
lupus patients.

In childhood lupus, researchers are evaluating the safety and effectiveness
of drugs called statins that lower LDL (or bad) cholesterol levels as a method
of preventing fat buildup in the blood vessels.

One out of five lupus patients experiences symptoms such as headaches, dizziness,
memory disturbances, stroke, or changes in behavior that result from changes
in the brain or other parts of the central nervous system. Such lupus patients
have what is called "neuropsychiatric" lupus. NIAMS-funded scientists are applying
new tools such as brain imaging techniques to discover cellular activity and
specific genes that may cause neuropsychiatric lupus. By uncovering the mechanisms
responsible for central nervous system damage in lupus patients, researchers
hope to move closer to improved diagnosis and treatment for patients with neuropsychiatric
lupus.

Researchers are focusing on finding better treatments for lupus. A primary
goal of this research is to develop treatments that can effectively minimize
the use of corticosteroids. Scientists are trying to identify combination therapies
that may be more effective than single treatment approaches. Another goal is
to improve the treatment and management of lupus in the kidneys and central
nervous system. For example, a 20- year study supported by the NIAMS and the
NIH found that combining cyclophosphamide with prednisone helped delay or prevent
kidney failure, a serious complication of lupus.

On the basis of new information about the disease process, scientists are
using novel "biologic agents" to selectively block parts of the immune system.
Development and testing of these new drugs, which are based on compounds that
occur naturally in the body, comprise an exciting and promising new area of
lupus research. The hope is that these treatments not only will be effective,
but also will have fewer side effects. Preliminary research suggests that white
blood cells known as B cells may play a key role in the development of lupus.
Biologics that interfere with B cell function or block the interactions of
immune cells are active areas of research. These targeted treatments hold promise
because they have the advantage of reduced side effects and adverse reactions
compared with conventional therapies. Clinical trials are testing the safety
and effectiveness of rituximab (also called anti-CD20) in treating people with
lupus. Rituximab is a genetically engineered antibody that blocks the production
of B cells. Other treatment options currently being explored include reconstructing
the immune system by bone marrow transplantation. In the future, gene therapy
also may play an important role in lupus treatment.

With research advances and a better understanding of lupus, the prognosis
for people with lupus today is far brighter than it was even 20 years ago.
It is possible to have lupus and remain active and involved with life, family,
and work. As current research efforts unfold, there is continued hope for new
treatments, improvements in quality of life, and, ultimately, a way to prevent
or cure the disease. The research efforts of today may yield the answers of
tomorrow, as scientists continue to unravel the mysteries of lupus.

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